Wednesday, September 8, 2010

Neurology Update

Yesterday we went to UNC to see the pediatric neurologist for Oliver's follow-up appointment. We were with the doc for close to 1 hour. There was so much information so I'll try not to bore you :)

We have noticed over the last week or so Oliver having “calm spells”, with sometimes a slight mouth twitch. The Dr. thinks these were seizures and increased Oliver's medication. He explained that the more seizures Oliver has, the higher the probability he will have more in the future, because seizures are something the brain learns to do over time. It is really important to control even these seemingly small/short, subtle seizures. Oliver’s medication (Tegretol) requires regular blood tests because one potentially serious side effect is losing significant amount of bone marrow. Unfortunately, all seizure meds have serious side effects. So far Oliver has done well with Tegretol.

We asked for an emergency seizure medication in case we are traveling and Oliver has a seizure that won't stop. We don’t anticipate this happening, but if it does we’d like to be prepared. The medication is no fun to administer, and if you give it you have to call 911 because in rare cases it can have serious respiratory side effects. Apparently, if you give it while on a flight the pilot needs to divert and land ASAP. Hopefully, we'll never have to use it and thankfully we don't plan to fly to Bali anytime soon :)

The Dr. checked the UNC records and found Oliver’s genetic testing results are still pending, so we have to try to keep being patient (which is really not my strong suit!).

For this next part you should understand the 3 types of brain tumors (always benign) you can have with TSC. This is taken from this webpagehttp://www2.massgeneral.org/livingwithtsc/affects/index.htmwhich has lots more if you want to read but, this is should be info for the purposes of this update.

"There are three main anatomical features associated with TSC that alter the structure of the brain: cortical tubers, subependymal nodules (SENs), and subependymal giant cell astrocytomas (SEGAs). Cortical tubers form in and around the cerebral cortex, the brain's outermost layer. SENs and SEGAs form deeper within the brain, typically along the ependymal lining (walls) of the ventricles, the cavities containing cerebrospinal fluid.

Like TSC lesions that affect other parts of the body, brain lesions associated with the disorder are composed of masses of abnormally shaped, dysfunctional cells. Tubers are composed of cells that fail to differentiate into functional neurons and glial cells during early stages of brain development. The resulting cell masses form before birth and are not thought to increase in size or number over time. In contrast, SENs and SEGAs are benign tumors composed of abnormal cells called neuroastrocytes, which remain in an interior region of the brain called the germinal layer, under the ependymal lining. As tumors, SENs and SEGAs can grow in both size and number over time.

SENs also differ from the relatively static tubers in that their growth can outpace that of the surrounding tissue, causing them to protrude into the cavities of the ventricles. While only 15 percent of SENs grow larger than 1 cm in diameter, those that do become classified as SEGAs and are cause for concern."

See pictures below. This ISN’T Oliver’s brain but, I will try to see if I can figure out how to upload his MRI and/or CT scan.

Alright, here is Oliver’s situation: Most of this was new information for us and it wasn’t what we were hoping for. We looked with the Dr. at the MRI and CT scans. We wanted to understand what the "3 calcifications" were on his brain. Those 3 spots were what they initially thought was bleeding. Until yesterday we only thought Oliver had 3 spots of abnormality/tumors. That is what we left UNC thinking. So I wanted to know, 1) Are the Tubers or SENs? and 2) Where are they located in the brain?…here was the news.

Unfortunately, those 3 spots are actually SENs that have calcium around them. We were so hoping they would be cortical tubers because they don’t grow into SEGAs. No one knows if Oliver’s 3 SENs will grow overtime or stay the same. Regardless, he has to be closely monitored. That means he will have an MRI annually (and more frequently if needed). We also found out that he also has at least 4 areas with cortical tubers. These tubers are what the disorder was named after. At that point the Dr. stopped and said “I don’t want to scare you (I think he didn’t want to look through the MRI/CT anymore). What matters the most with Oliver is, what you see is what you get. Even though he has these lesions, if he is doing well, focus on that. If he isn’t doing well, they will worry more about the tumors.” So that made us feel a bit better, but honestly this all was a big shock to us.

2 Corinthians 4:8-9 We are hard pressed on every side, but not crushed; perplexed, but not in despair; persecuted, but not abandoned; struck down, but not destroyed.

Thank you for walking with us on this journey! We are grateful for your prayers for us as we continue to try to glorify God in this challenging time. Also, please pray for unity and peace for us as we consider how to go about having more children.

Oliver has 3 SENs in the lateral ventricle

Oliver DOES NOT (Thank God) have a SEGA and hopefully never will!


Oliver does have at least 4 cortical tubers

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